Thibaud Damy1,2,3,4,5,6, Diane Bodez1,2,3,4,5,6, Anoosha Habibi4,7, Aziz Guellich1,2,3,4,5,6, Stéphane Rappeneau1,2,3,4,5,6, Jocelyn Inamo8, Soulef Guendouz1,2,3,4,6, Justine Gellen-Dautremer7, Serge Pissard9, Sylvain Loric10, Orianne Wagner-Ballon11, Bertrand Godeau2,12, Serge Adnot13,14, Jean-Luc Dubois-Randé1,2,3,4, Luc Hittinger1,2,3,4, Frédéric Galactéros2,4,7,12,15, Pablo Bartolucci2,4,7,12,15. 1. AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France. 2. School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France. 3. IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France. 4. DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France. 5. INSERM Clinical Investigation Centre 1430, Créteil F-94000, France. 6. Mondor Amyloidosis Network, Créteil F-94000, France. 7. AP-HP, UMGGR, Henri Mondor Teaching Hospital, Creteil F-94000, France. 8. Department of Cardiology, Martinique Teaching Hospital, Fort-de-France 97200, France. 9. AP-HP, Department of Genetics, Henri Mondor Teaching Hospital, Creteil F-94000, France. 10. AP-HP, Department of Biochemistry, Henri Mondor Teaching Hospital, Creteil F-94000, France. 11. AP-HP, Department of Biological Hematology and Immunology, Henri Mondor Teaching Hospital, Creteil F-94000, France. 12. AP-HP, Department of Internal Medicine, Henri Mondor Teaching Hospital, Creteil F-94000, France. 13. AP-HP, Department of Physiology, Henri Mondor Teaching Hospital, Creteil F-94000, France. 14. IMRB INSERM U955, Team 8, Paris-Est University, UPEC, France. 15. IMRB INSERM U955, Team 2, Paris Est University, UPEC, France.
Abstract
AIMS: Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis. METHODS AND RESULTS: This longitudinal observational study was performed on 1780 SCD patients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained <1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI) >4 L/min/m(2), LV ejection fraction <55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction <55% was present also (P = 0.0001). CONCLUSION: Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis. METHODS AND RESULTS: This longitudinal observational study was performed on 1780 SCDpatients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained <1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI) >4 L/min/m(2), LV ejection fraction <55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction <55% was present also (P = 0.0001). CONCLUSION:Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality. Published on behalf of the European Society of Cardiology. All rights reserved.
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